Role of site-1 and site-2 protease for lysosomal homeostasis (B03)

• Subject Area: Biochemistry
• Term: from 2010 to 2022
• Project identifier: Deutsche Forschungsgemeinschaft (DFG) - Project number 125440785

The project aims to identify ER proteins interacting with the inactive α/β-subunit precursor protein of GlcNAc-1-phosphotransferase that requires proteolytic activation by the Golgi-resident site-1 protease (S1P) but not site-2 protease (S2P). Still, absence of S2P leads to missorting of lysosomal enzymes and accumulation of lysosomal storage material. Mutations in the S2P gene result in osteogenesis imperfecta, a collagen-related bone disorder. The project will analyze S2P knock-in mice and the role of IL-6 in progressive bone loss in GlcNAc-1-phosphotransferase-inactive mice.

• DFG Programme: Collaborative Research Centres
• Subproject of: SFB 877: Proteolysis as a Regulatory Event in Pathophysiology
• Applicant Institution: Christian-Albrechts-Universität zu Kiel
• Project Heads: Professor Dr. Thomas Braulke; Professorin Dr. Sandra Pohl